Case Report: Combined perioperative extracorporeal membrane oxygenation for acute heart failure caused by mitral regurgitation.

Extracorporeal membrane oxygenation (ECMO) and extracorporeal life support (ECLS) devices are well-established adjunctive treatment measures for patients with heart failure. ECMO can serve as a bridge to transplant in a chronic setting or as a salvage therapy for patients who are unable to be weaned from bypass following cardiac surgery. However, the role of ECMO as a bridge to definitive therapy in a setting of acute heart failure is less established. Similarly, the treatment of patients using combined ECMO and ECLS devices has been, at times, shown to show some benefit; however, these benefits have not been widely studied. In this study, we present the case of a patient who was diagnosed with severe acute onset heart failure secondary to torrential mitral regurgitation following COVID-19 pneumonia. The patient was emergently placed on venoarterial (VA) ECMO with an indwelling centrifugal pump device in the left ventricle. This combination of ECMO and ECLS served as a bridge to open mitral valve replacement 6 days after presentation. Following successful mitral valve replacement, the patient had persistent right ventricular failure, and therefore, a decision was made to incorporate venovenous (VV) ECMO into the VA ECMO circuit. This technique resulted in a VV-VA or VPa-VA configuration, as oxygenated blood was being returned to the pulmonary artery as well as the descending aorta. VA ECMO was discontinued after 4 days of therapy, and the patient was extubated 3 days later. VV ECMO was weaned over the following week, and the patient was decannulated after a total 23 days of ECMO. The patient was then transitioned to inpatient rehabilitation and ultimately discharged home after 18 days. At the 6-month follow-up, the patient was doing well, and objective cardiopulmonary testing revealed normal function. This case is an excellent demonstration of how advanced ECMO and ECLS devices can be used in unique ways through multiple configurations to rescue and optimize patients in the perioperative period.

Impact of pulmonary hypertension on outcomes after TEER in patients suffering from mitral regurgitation.

AIM: Data on associations of invasively determined hemodynamic parameters with procedural success and outcomes in patients suffering from mitral regurgitation (MR) undergoing transcatheter edge-to-edge repair of the mitral valve (M-TEER) is limited. METHODS AND RESULTS: We enrolled 239 patients with symptomatic MR of grade 2 + , who received M-TEER. All patients underwent extensive pre-interventional invasive hemodynamic measurements via right heart catheterization (mean pulmonary arterial pressure (mPAP), systolic- (PAPsys) and diastolic pulmonary arterial pressure (PAPdia), pulmonary arterial wedge pressure (PAWP), a-wave, v-wave, pulmonary vascular resistance (PVR), transpulmonary pressure gradient (TPG), cardiac index (CI), stroke volume index (SVI)). mPAP and PAWP at baseline were neither associated with procedural success, immediate reduction of MR, nor residual MR after 6 months of follow-up. The composite outcome (All-cause mortality (ACM) and/or heart failure induced rehospitalization (HFH)) and HFH differed significantly after M-TEER when stratified according to mPAP, PAWP, PAPdia, a-wave and v-wave. ACM was not associated with the afore mentioned parameters. Neither PVR, TPG, CI nor SVI were associated with the composite outcome and HFH, respectively. In multivariable analyses, PAWP was independently associated with the composite outcome and HFH. PVR and SVI were not associated with outcomes. CONCLUSION: PAWP at baseline was significantly and independently associated with HFH and might serve as a valuable parameter for identifying patients at high risk for HFH after M-TEER. ACM and procedural success were not affected by pulmonary arterial pressure before M-TEER. We suggest that the post-capillary component of PH serves as the driving force behind the risk of HFH.

Telesimulation Training for Endoscopic Mitral Valve Surgery: An Air-Pilot Training Concept for Distance Training.

OBJECTIVE: The aim of this study was to validate and assess the feasibility and impact of telesimulation training on surgical skills using a portable mitral valve telesimulator. METHODS: A telesimulation course composed of 3 online modules was designed based on backwards chaining, preassessment and postassessment, performance feedback, hands-on training on a telesimulator, and the theoretical content. A fully 3-dimensional-printed and transportable telesimulator was developed and sent out to the participants with instruments that were needed. Feedback about the platform was obtained from participants to validate its value as a training tool. Theoretical and technical assessments were carried out before and after the course. Technical assessments were based on the accuracy and time taken to place sutures at the anterior and posterior mitral annulus. RESULTS: In total, 11 practicing cardiac surgeons from Oceania, Asia, Europe, and North America completed the course. Theoretical preassessment and postassessment showed that participants scored significantly higher on postassessment (mean 87.5% vs 68.1%, P < 0.004). The participant evaluation scores of the simulator as a tool for endoscopic mitral valve surgery was 4 to 5 out of 5. There was a significant improvement in the speed (median 14.5 vs 39.5 s, P < 0.005) and the accuracy to place sutures in the mitral valve annulus following course completion (P < 0.001). CONCLUSIONS: Here we validated the educational value of a novel telesimulation platform and validated the feasibility to teach participants at a distance the knowledge and skills for endoscopic mitral valve surgery. Future studies will be required to validate the improvement in skills during surgery.

Proteomic analysis of the serum in dogs with pulmonary hypertension secondary to myxomatous mitral valve disease: the preliminary study.

Background: Pulmonary hypertension (PH) is a common complication in dogs with myxomatous mitral valve disease (MMVD), characterized by elevated blood pressure in pulmonary artery. Echocardiography is a reliable technique for PH diagnosis in veterinary medicine. However, it is limited to use as an early detection method. Liquid chromatography-tandem mass spectrometry (LC-MS/MS) has found extensive application in the discovery of serum protein biomarkers for various diseases. The objective of this study was to identify serum proteins in healthy control dogs and MMVD dogs both with and without PH using LC-MS/MS. Materials and methods: In this research, a total of 81 small-breed dogs participated, and they were categorized into three groups: the control (n = 28), MMVD (n = 24) and MMVD+PH (n = 29) groups. Serum samples were collected and analyzed by LC-MS/MS. Results: Differentially expressed proteins were identified, and the upregulated and downregulated proteins in MMVD+PH group including Myomesin 1 (MYOM1) and Histone deacetylase 7 (HDAC7), Pleckstrin homology domain containing M3 (PLEKHM3), Diacylglycerol lipase alpha (DAGLA) and Tubulin tyrosine ligase like 6 (TTLL6) were selected as proteins of interest in MMVD dogs with PH. Conclusion: Different types of proteins have been identified in healthy dogs and MMVD dogs with and without PH. Additional studies are needed to investigate the potential of these proteins as biomarkers for PH in dogs with MMVD.

Paradox of disproportionate atrial functional mitral regurgitation and survival after transcatheter edge-to-edge repair.

AIMS: This study aims to assess the applicability of the mitral regurgitation (MR) proportionality concept in patients with atrial functional mitral regurgitation (aFMR) treated with transcatheter edge-to-edge repair (M-TEER). We hypothesized that patients with disproportionate MR (higher MR relative to left ventricular size) would exhibit different outcomes compared to those with proportionate MR, despite undergoing M-TEER. METHODS AND RESULTS: We retrospectively analysed 98 patients with aFMR from the EuroSMR registry who underwent M-TEER between 2008 and 2019. Patients met criteria for aFMR (normal indexed left ventricular end-diastolic volume [LVEDV], preserved left ventricular ejection fraction [LVEF] ≥ 50% without regional wall motion abnormalities, and structurally normal mitral valves). We excluded patients with missing effective regurgitant orifice area (EROA) or LVEDV data. The primary endpoint was 2-year mortality, with an EROA/LVEDV ratio employed to differentiate disproportionate from proportionate MR. Procedural success and baseline characteristics were analysed, and multivariate Cox proportional hazards models were used to identify mortality predictors. The mean patient age was 79 ± 7.3 years, with 68.8% female, and 79% had a history of atrial fibrillation. The mean EROA was 0.27 ± 0.14 cm2, and LVEDV was 95.6 ± 33.7 mL. Disproportionate MR was identified with an EROA/LVEDV ratio >0.339 cm2/100 mL. While procedural success was similar in both groups, disproportionate MR was associated with a numerically higher estimate of systolic pulmonary artery pressures (sPAP) and rates of NYHA ≥III and TR ≥ 3+. Disproportionate MR had a significant association with increased 2-year mortality (P < 0.001). The EROA/LVEDV ratio and tricuspid annular plane systolic excursion (TAPSE) were independent predictors of 2-year mortality (EROA/LVEDV: HR: 1.35, P = 0.010; TAPSE: HR: 0.85, P = 0.020). CONCLUSIONS: This analysis introduces the MR proportionality concept in aFMR patients and its potential prognostic value. Paradoxically, disproportionate MR in aFMR was linked to a 1.35-fold increase in 2-year mortality post-M-TEER, emphasizing the importance of accurate preprocedural FMR characterization. Our findings in patients with disproportionate MR indicate that a high degree of aFMR with high regurgitant volumes may lead to aggravated symptoms, which is a known contributor to increased mortality following M-TEER. These results underline the need for further research into the pathophysiology of aFMR to inform potential preventative and therapeutic strategies, ensuring optimal patient outcomes.

ACTN1-related thrombocytopenia: Homozygosity for an ACTN1 variant results in a more severe phenotype.

ACTN1-related thrombocytopenia is a rare disorder caused by heterozygous variants in the ACTN1 gene characterized by macrothrombocytopenia and mild bleeding tendency. We describe for the first time two patients affected with ACTN1-RT caused by a homozygous variant in ACTN1 (c.982G>A) with mild heart valve defects unexplained by any other genetic variants investigated by WES. Within the reported family, the homozygous sisters have moderate thrombocytopenia and marked platelet macrocytosis with giant platelets, revealing a more severe haematological phenotype compared to their heterozygous relatives and highlighting a significant effect of allelic burden on platelet size. Moreover, we hypothesize that some ACTN1 variants, especially when present in the homozygous state, may also contribute to the cardiac abnormalities.

[Catheter-based and surgical treatment of mitral valve diseases]. / Katheterbasierte und operative Therapie bei Mitralklappenvitien.

There is a broad spectrum of mitral valve diseases ranging from young patients with rheumatic mitral valve stenosis up to older patients with secondary mitral valve regurgitation and numerous comorbidities. A profound understanding of the etiology, anatomical characteristics of mitral valve diseases and current treatment options is necessary to be able to prepare a patient-centered treatment approach. The interdisciplinary collaboration of referring physicians, interventional cardiologists, cardiac surgeons, heart failure and imaging specialists as well as anesthesiologists is a cornerstone of optimal patient treatment.

Transcatheter mitral valve repair with MitraClip: comparison of NT, NTr, and XTr Devices.

OBJECTIVES: Transcatheter edge-to-edge repair (TEER) has become an established minimally invasive treatment for significant mitral regurgitation. Ongoing refinements and the availability of different clipping devices have expanded the indications for and effectiveness of TEER, but comprehensive comparative data on this issue are lacking. In this study, we compared NT, NTr, and XTr MitraClip devices (Abbot) for TEER. METHODS: Details on patient, imaging, and procedural details, as well as short- and long-term outcomes, were sought from a national prospective clinical registry on TEER with MitraClip. The primary outcome of interest was discharge after procedural success without major clinical complications. RESULTS: A total of 2236 patients were included, 1228 (54.9%) in whom NT implantation only was attempted, 233 (10.4%) in whom NTr but not XTr implantation was attempted, and 775 (34.7%) in whom XTr implantation was attempted. Clinical and imaging features differed substantially across the groups, reflecting expanding indications with NTr and XTr devices. In-hospital outcomes were largely similar among the 3 groups, including death. Long-term unadjusted estimates of effect showed significant differences in several outcomes, including death, rehospitalization, and their composite, which demonstrated that NT was associated with more unfavorable outcomes compared with the other devices (all P less than .05). However, most differences depended on baseline features, as adjusted analysis showed no significant differences for early as well as long-term outcomes, including long-term death, rehospitalization, and their composite (all P greater than .05). CONCLUSIONS: New-generation MitraClip devices are associated with favorable procedural and clinical outcomes, despite being used in patients with more adverse features, when compared with patients treated with previous devices.

Optimal patient selection for maze procedure in patients undergoing mitral valve disease.

OBJECTIVES: Although risk factors for unsuccessful Maze procedure have been demonstrated, an appropriate patient selection is still controversial. In our institute, Maze procedure is indicated for those whom normal sinus rhythm (NSR) was reestablished by intraoperative direct cardioversion (DC) after ventricular unloading by total cardiopulmonary bypass. The purpose of this study was to evaluate the effectiveness of our indication criteria for Maze procedure in patients with mitral valve disease. METHODS: Between October 2012 and October 2021, MAZE was indicated in 55 patients in whom normal sinus rhythm (NSR) was reestablished by intraoperative direct current cardioversion (DC). Three endpoints and predictors were examined: disappearance of atrial fibrillation (AF), NSR, and A-wave detection. RESULTS: Restoration of NSR by intraoperative DC was confirmed in 43 patients, and these patients underwent MAZE. AF disappeared in 39 patients (90.7%), and F-wave ≥ 0.1 mV was a significant predictive factor (odds ratio (OR) 20.99, 95% CI 1.22-1079.06). NSR was reestablished in 36 patients (83.7%), and F-wave ≥ 0.1 mV (odds ratio 15.62, 95% CI 1.62-359.86) + AF history ≤ 3 years (OR 8.30, 95% CI 1.09-177.04) were significant predictors. A-wave detection was confirmed in 26 patients (60.5%), and left atrial diameter ≤ 55 mm was a significant predictor (OR 5.22, 95% CI 1.28-24.79). CONCLUSIONS: Intraoperative DC after ventricular unloading resulted effective patient selection for concomitant Maze procedure. F-wave and AF history were predictive factor of electrical restoration of AF, and left atrial diameter was predictive factor of restoration of atrial function.

Contemporary 1-Year Outcomes of Mitral Valve-in-Ring With Balloon-Expandable Aortic Transcatheter Valves in the U.S.

BACKGROUND: Adequate valve performance after surgical mitral valve repair with an annuloplasty ring is not always sustained over time. The risk of repeat mitral valve surgery may be high in these patients. Transcatheter mitral valve-in-ring (MViR) is emerging as an alternative for high-risk patients. OBJECTIVES: The authors sought to assess contemporary outcomes of MViR using third-generation balloon-expandable aortic transcatheter heart valves. METHODS: Patients who underwent MViR and were enrolled in the STDS/ACC TVT (Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy) Registry between August 2015 and December 2022 were analyzed. RESULTS: A total of 820 patients underwent MViR at 236 sites, mean age was 72.2 ± 10.4 years, 50.9% were female, mean STS score was 8.2% ± 6.9%, and most (78%) were in NYHA functional class III to IV. Mean left ventricular ejection fraction was 47.8% ± 14.2%, mean mitral gradient was 8.9 ± 7.0 mm Hg, and 75.5% had ≥ moderate mitral regurgitation. Access was transseptal in 93.9% with 88% technical success. All-cause mortality at 30 days was 8.3%, and at 1 year, 22.4%, with a reintervention rate of 9.1%. At 1-year follow-up, 75.6% were NYHA functional class I to II, Kansas City Cardiomyopathy Questionnaire score increased by 25.9 ± 29.1 points, mean mitral valve gradient was 8.4 ± 3.4 mm Hg, and 91.7% had ≤ mild mitral regurgitation. CONCLUSIONS: MViR with third-generation balloon-expandable aortic transcatheter heart valves is associated with a significant reduction in mitral regurgitation and improvement in symptoms at 1 year, but with elevated valvular gradients and a high reintervention rate. MViR is a reasonable alternative for high-risk patients unable undergo surgery who have appropriate anatomy for the procedure. (STS/ACC TVT Registry Mitral Module [TMVR]; NCT02245763).

The added value of three-dimensional transthoracic echocardiography in mitral annular disjunction: a case report.

Background: Mitral annular disjunction (MAD) refers to the arrhythmic mitral valve prolapse (MVP) syndrome associated with ventricular arrhythmias and sudden cardiac death. Although the pathophysiology of this disease is still under investigation, specific imaging criteria that establish the diagnosis have been recognized. In this article, we demonstrate most of these criteria using three-dimensional transthoracic echocardiography (3D-TTE) and provide added value in the management of MAD syndrome. Case presentation: A 50-year-old male patient with recent syncope and a history of mitral regurgitation (MR) and MAD was admitted to our clinic for further investigation. According to our protocol, the patient underwent a complete 3D-TTE, laboratory blood exams, and 24 h ambulatory electrocardiogram (ECG). Our investigation confirmed the presence of MAD syndrome with bileaflet prolapse, severe MR, and non-sustained ventricular tachycardia, necessitating an implantable cardioverter defibrillator (ICD) and surgical mitral valve repair. The 3D-TTE analysis of the mitral valve demonstrated mitral annular systolic expansion and systolic flattening of the saddle-shaped annulus and quantified the extent of the disjunction arc. Additionally, four-dimensional (4D) strain analysis of the left ventricle revealed the presence of fibrosis of the posteromedial papillary muscle and basal inferolateral wall, which are variables that are required for the diagnosis and therapeutic management of MAD syndrome. Conclusions: 3D-TTE and 4D strain offer valuable insights for diagnosing and managing patients with MAD syndrome. This method seems to correlate well with the other imaging modalities and could be included in the management protocol of MAD syndrome.

Transcatheter Mitral Valve Repair Using a Novel Device for a High-Surgical-Risk Patient With DMR.

Transcatheter edge-to-edge repair (TEER) has been established as a safe and effective option for treating patients with severe symptomatic degenerative mitral regurgitation (MR) who are at prohibitive surgical risk. However, the significant cost presents a considerable disease burden in low-income countries. This case details the treatment of a high-surgical-risk patient with severe degenerative MR by using the GeminiOne (Peijia Medical) system-a novel Chinese TEER device.

Revisiting the anatomy of the left ventricle in the light of knowledge of its development.

Despite centuries of investigation, certain aspects of left ventricular anatomy remain either controversial or uncertain. We make no claims to have resolved these issues, but our review, based on our current knowledge of development, hopefully identifies the issues requiring further investigation. When first formed, the left ventricle had only inlet and apical components. With the expansion of the atrioventricular canal, the developing ventricle cedes part of its inlet to the right ventricle whilst retaining the larger parts of the cushions dividing the atrioventricular canal. Further remodelling of the interventricular communication provides the ventricle with its outlet, with the aortic root being transferred to the left ventricle along with the newly formed myocardium supporting its leaflets. The definitive ventricle possesses inlet, apical and outlet parts. The inlet component is guarded by the mitral valve, with its leaflets, in the normal heart, supported by papillary muscles located infero-septally and supero-laterally. There is but a solitary zone of apposition between the leaflets, which we suggest are best described as being aortic and mural. The trabeculated component extends beyond the inlet to the apex and is confluent with the outlet part, which supports the aortic root. The leaflets of the aortic valve are supported in semilunar fashion within the root, with the ventricular cavity extending to the sinutubular junction. The myocardial-arterial junction, however, stops well short of the sinutubular junction, with myocardium found only at the bases of the sinuses, giving rise to the coronary arteries. We argue that the relationships between the various components should now be described using attitudinally appropriate terms rather than describing them as if the heart is removed from the body and positioned on its apex.

Clinical Impact of the Endo-aortic Clamp for Redo Mitral Valve Surgery.

Aim of this study was to compare redo MV surgery patients undergoing right mini-thoracotomy and EAC with redo MV patients undergoing surgery through other approaches. Redo MV patients from 7 European centers were analyzed. Primary endpoint was 30-day mortality; secondary endpoints were stroke, re-exploration, low cardiac output syndrome (LCOS), respiratory failure, and intensive care unit (ICU) and in-hospital length-of-stay. Forty-nine patients underwent right mini-thoracotomy and EAC (22.7%), and 167 (77.3%) underwent surgery through other approaches (112 sternotomy, 40 unclamped mini-thoracotomies, and 15 mini-thoracotomies with trans-thoracic clamp). Thirty-day mortality, stroke, re-exploration for bleeding, and weaning failure were comparable. The EAC group showed significant lower rate of LCOS (p = 0.03) and shorter ICU (p = 0.04) and in-hospital length of stay (p = 0.002). The EAC allows the surgeon to reach the aorta, to clamp it, and to deliver the cardioplegia with a "no-touch" technique, with significant improvement in outcomes.

Left Atrial Enlargement as a Predictor of Atrial Fibrillation in Rheumatic Mitral Valve Disease: An Echocardiography-based Retrospective Cross-sectional Study.

BACKGROUND: Rheumatic heart disease is a major cause of cardiovascular morbidity and mortality in developing nations and is a leading cause of hospital admission due to cardiac problems in our country. This study will evaluate the association between left atrial size and the occurrence of atrial fibrillation and describe the clinical characteristics along with complications related to Rheumatic Mitral valve disease. METHODS: A retrospective cross-sectional study was conducted at a tertiary care center from January 2018 to December 2019. Reports of 207 patients admitted to medical and/or surgical wards with echocardiographic diagnosis of rheumatic mitral valve disease with or without atrial fibrillation were reviewed. Data were collected, entered, and analyzed using the Statistical Package for the Social Science version 25.0. RESULTS: Among 207 patients, atrial fibrillation was present in 90 (43.5%) patients. Atrial fibrillation was higher in patients with mixed mitral valvular lesions compared to isolated mitral stenosis or mitral regurgitation. Univariate and multivariate analysis revealed left atrial size [aOR=1.067, 95% CI: 1.023 - 1.113, P= 0.002] and age [aOR = 1.073, 95% CI: 1.042 - 1.105, P<0.001] as an independent predictor of atrial fibrillation. CONCLUSIONS: Larger left atrium was an independent predictor of atrial fibrillation. Besides this, atrial fibrillation was associated with increasing age, mixed mitral valvular lesion, and moderately reduced left ventricular ejection fraction, but not associated with gender and mitral stenosis severity. Left atrial clot was significantly higher in patients with atrial fibrillation than in sinus rhythm.

Clinical and echocardiographic results of the MEMO 4D semi-rigid annuloplasty ring.

BACKGROUND: Mitral regurgitation is a frequent valvular disease, with an increasing prevalence. We analyzed the short-term outcomes of mitral valve repair procedures conducted in our clinic using a new semirigid annuloplasty ring featuring a gradual saddle shape design. METHODS: We retrospectively analyzed mitral valve repair surgeries performed at our Institution between December 2019 and November 2021 with the MEMO 4D semirigid annuloplasty ring. RESULTS: In total, 53 patients were included in the study. Mean patient age was 63.6 ± 11.7 years. Most patients presented with degenerative mitral valve regurgitation (N = 44; 83%). The grade of mitral regurgitation was equal or more than 3 + in 98.1% of the patients (N = 52). The most used ring size was size 34 mm (N = 30, 56.6%). There was no intraoperative or hospital mortality. No cases of stroke, bleeding, endocarditis or other major complications occurred. At discharge, most patients were in NYHA class I. Postoperative echocardiographic results showed no (90.6%) or 1+ (5.7%) mitral valve regurgitation. Only 1 patient (1.9%) presented with mitral valve regurgitation grade 2+. Mean postoperative transvalvular gradient was low (mean = 3.3 ± 1.2 mmHg). No cases of LVOT obstruction or systolic anterior motion occurred. CONCLUSIONS: Our series showed excellent mitral valve competency and very satisfactory early clinical outcomes. The transesophageal echocardiographic follow-up, despite obtained in a limited number of patients, further confirmed the effectiveness of findings of this preliminary experience.